The correction of Hunter fibroblasts by exogenous iduronate sulfate sulfatase: biochemical and ultrastructural studies.
The exogenous addition of iduronate sulfate sulfatase to cultured fibroblasts of Hunter patients resulted in a full correction of the metabolic defect as demonstrated by chemical and ultrastructural analyses. As little as 25% of the normal fibroblasts' enzyme levels were sufficient for this correction. The half-disappearance time of the internalized enzyme was 3-4 days. Prolonged incubation of corrected cells resulted in a gradual reaccumulation of mucopolysaccharides.
D002478:Cells, Cultured; D005347:Fibroblasts / Q000648:ultrastructure*; D006801:Humans; D007066:Iduronate Sulfatase; D066298:In Vitro Techniques; D008854:Microscopy, Electron; D016532:Mucopolysaccharidosis II / Q000628:therapy; D013429:Sulfatases
D013429:Sulfatases; D007066:Iduronate Sulfatase